Informed consent and hypoplastic left heart syndrome

Abstract

We present an ethical analysis from the perspective of shared decision-making and informed consent of a change in clinical management of infants born with hypoplastic left heart syndrome (HLHS). We reported a change in treatment of HLHS at the University of Alberta away from comfort care to life-saving surgery (LST) between 1987 and 1998. In a second review (1996–2001), 49/62 infants received LST, with 81% survival from the NICU and 58% at 35 mo. Eleven infants died preoperatively of non-cardiac conditions and two received elective comfort care. Sixteen infants had 18-mo Bayley Mental Development Index, mean score 84±19, but five scored <70. Although we continue to present the comfort care option to parents, since 2001 LST use for HLHS at our center is almost universal despite serious complications. Conclusion: We conclude that these findings are inconsistent with an open, shared decision-making model of informed consent and we suggest that comfort care should remain an ethically valid choice until the rate of serious long-term complications of LST decreases.